Gray platelet syndrome
Other Names for this Disease
- Platelet alpha-granule deficiency
- Marked decrease or absence of alpha-granules and of platelet-specific alpha-granule proteins
- Alpha storage pool deficiency
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There is no specific treatment for GPS, but management involves anticipating and preventing risks of bleeding (e.g. possible platelet transfusions before surgery). Treatment may also include administration of desmopressin. Splenectomy should be considered to increase the platelet counts in those whose platelet counts decrease to approximately 30,000/microliter. Prognosis is generally good early in life when thrombocytopenia is mild. Those with platelets counts less than 30,000/microliter are at risk for life-threatening bleeding.
Last updated: 10/3/2011
- Gunay-Aygun M & Gahl WA. Gray platelet syndrome. Orphanet. June 2011; http://www.orpha.net/consor/cgi-bin/OC_Exp.php?lng=EN&Expert=721. Accessed 10/3/2011.
- Finding Treatment Information - A video developed by GARD Information Specialists that explains how you can find information about treatment for a rare disease.
- ClinicalTrials.gov lists trials that are studying or have studied Gray platelet syndrome. Click on the link to go to ClinicalTrials.gov to read descriptions of these studies.